Epidermolysis bullosa

As it has already been mentioned, itchiness is a very complex problem especially frequent in the severe types of EB. In some studies, the people suffering from it describe it as the most problematic symptom, even before pain. Its frequency usually increases as the patients describe their global affectation of the disease is more severe.

There are not prevalence consistent data, but in some cases, it can present in more than 80% of the patients. Although it can appear in in all the types and sub-types of EB, the highest prevalence is found in patients with junctural EB, recessive dystrophic BE, dominant dystrophic EB, simple EB and severe simple EB.

The patients with pruritic DEB (P-DEB) deserve a special mention as, although it is a rare sub-type, the itchiness is extremely severe and it can appear from birth, also associated to specific skin injuries, especially in the pretibial area. P-DEB is characterized by an intense itch, with injuries usually situated on the lower part of the lower extremities and forearmsand altered nails. The injuries tend to be more evident some years after birth (2nd - 3rd life stage). This type of EB can be confused with other diseases.